It has been proposed that epithelial dysfunction and inflammation may predispose patients to kidney stone formation. Asthma is another chronic condition related to epithelial dysfunction and inflammation. We hypothesized that pediatric patients with asthma would have an increased prevalence of nephrolithiasis. Furthermore, we investigated if asthma patients with nephrolithiasis have clinical characteristics and urine profiles that point to mechanisms of stone formation. We evaluated 865 pediatric patients who had a diagnosis of nephrolithiasis. Clinical/demographic data and 24 hour urine samples were compared between asthma + stone (n = 142) and stone only patients. Data from asthmatics without stone were also available for evaluation of medication differences among asthma + stone and asthma only patients. The prevalence of nephrolithiasis in the pediatric population at our institution was 0.08% vs. 0.31% in our pediatric asthmatic population. The prevalence of asthma in our pediatric population was 6.8% vs. 26.7% in our pediatric stone patients. Asthma + stone patients were more likely to be on a combination inhaled corticosteroid + long acting beta agonist inhaler as compared to age/gender/BMI matched asthma patients without stone (29.7% vs. 13.7%, p = 0.0012). 259 kidney stone patients had 24 hour urine samples for comparison. There was no difference in 24 hour urine profiles between asthma + stone and stone only patients. Children with asthma have a 4-fold greater prevalence of kidney stones than the general pediatric population. Similarly, children with kidney stones have a 4-fold greater prevalence of asthma. This correlation may suggest a mechanistic link between asthma and nephrolithiasis. Further investigation is needed to elucidate the pathophysiologic origin of this relationship.